Epidemiology of rheumatic heart disease among primary school children in western Kenya

A community survey was conducted to determine the prevalence of rheumatic heart disease in Kenyan children in all primary schools in 2 sublocations in Western Kenya. In total, 3631 children (97% school attendance) aged from 5 to 15 years were examined by one experienced physician and 2 cardiologists between September and December 1985. Six children were found to have evidence of rheumatic heart disease, giving a prevalence rate of 1.7/1000. As all cases occurred in the age group between 10 and 15 years, the rate was calculated for this group as 2.4/1000. None of these patients had previously been diagnosed as having rheumatic fever. There were 3 males and 3 females. Four patients had mitral regurgitation, one had aortic regurgitation and one had mitral stenosis. Three had electrocardiographic evidence of left atrial abnormality and one showed findings of left ventricular hypertrophy but two patients had normal tracings. Half were symptomatic with dyspnoea on exertion and two gave a history of fleeting polyarthritis. The socio-economic status (peasant), family size (mean = 6 siblings) and the number of children sleeping per room were the same in both the cases with rheumatic heart disease and their controls. The health workers achieved low scores (mean 26%) with regard to the clinical diagnosis, treatment and prevention of rheumatic fever at the level of primary health care. Rheumatic heart disease, nonetheless, was relatively insignificant in the overall picture of health care of school children in Western Kenya.     

Prevalence of congenital heart disease in black schoolchildren of Soweto, Johannesburg.

A survey conducted by cardiologists in Soweto, Johannesburg, provided an opportunity of assessing the frequency of congenital heart disease in black schoolchildren. Among 12,050 schoolchildren aged 2 to 18 years, 48 had a congenital heart defect, yielding a prevalence of 3.9 per 1000. Only in 2- to 6-year-old children did the prevalence exceed that of rheumatic heart disease. The distribution of the types of defects was largely similar to that reported in other surveys with a predominance (52%) of ventricular septal defects. Two unusual findings were the unexplained absence of persistent ductus arteriosus in these children, and the detection of 5 children with situs inversus (1 in 2410). In all but one child, the congenital heart defect was first discovered during the survey. Despite the limitations of a prevalence study, it can be concluded that congenital heart disease is at least as common in this South African black community as in Caucasians.     

Prevalence of congenital heart disease in black schoolchildren of Soweto, Johannesburg.

A survey conducted by cardiologists in Soweto, Johannesburg, provided an opportunity of assessing the frequency of congenital heart disease in black schoolchildren. Among 12,050 schoolchildren aged 2 to 18 years, 48 had a congenital heart defect, yielding a prevalence of 3.9 per 1000. Only in 2- to 6-year-old children did the prevalence exceed that of rheumatic heart disease. The distribution of the types of defects was largely similar to that reported in other surveys with a predominance (52%) of ventricular septal defects. Two unusual findings were the unexplained absence of persistent ductus arteriosus in these children, and the detection of 5 children with situs inversus (1 in 2410). In all but one child, the congenital heart defect was first discovered during the survey. Despite the limitations of a prevalence study, it can be concluded that congenital heart disease is at least as common in this South African black community as in Caucasians.     

Prevalence of rheumatic fever and rheumatic heart disease in school children in a rural community of the hill region of Nepal

A survey of school children aged 5 to 16 years living in a rural community of the hill region of Nepal, situated about 15-22 km outside Kathmandu city, was conducted to determine the prevalence of rheumatic fever (RF) and rheumatic heart disease (RHD). Of the 4,816 eligible children enrolled in the selected schools, 4,452 (92.4%) were examined. WHO expert committee criteria (1966) was used for the diagnosis and classification of rheumatic fever. Chest x-ray, electro-cardiography, echocardiography and Doppler study were done in all suspected cases of rheumatic heart disease. Six cases of RHD (1 pure mitral stenosis, 3 mitral regurgitation and 2 combined mitral stenosis and regurgitation) were identified giving overall prevalence rate of 1.35 per thousand. No case with active rheumatic fever could be identified. This is the first study on prevalence of RF/RHD in Nepal. The prevalence rate is lower than that reported from neighbouring countries.     

Atrial septal defect (secundum) associated with mitral regurgitation

Mitral regurgitation was found in 6 percent (46 patients) of all patients with a secundum atrial septal defect who were operated on during a 13 year period at the Mayo Clinic. The presence of two separate systolic murmurs, expiratory splitting of the second heart sound and evidence of overactivity of both the right and the left ventricles were the most reliable clues to the diagnosis. Nineteen of 39 patients (49 percent) had increased pulmonary arterial pressure, but only 3 patients had obstructive pulmonary vascular disease. Electrocardlographic findings rarely helped differentiate the patient who had a secundum atrial septal defect and associated mitral regurgitation from the one who had an isolated septal defect. The causes for the mitral regurgitation varied. Six patients had rheumatic valvulitis, 8 had redundant mitral valve tissue with prolapsing leaflets, 9 patients had two or more causes, and in 18, no cause for the regurgitation could be found.     

Secundum atrial septal defect and significant mitral regurgitation: incidence, management and morphologic basis.

To better understand the association between mitral regurgitation and secundum atrial septal defect and to clarify the evaluation and management of these patients, the records of 235 adult patients with atrial septal defect were reviewed. Ten patients (4 percent) had significant mitral regurgitation defined by clinical, hemodynamic and angiographic criteria. Three patients required mitral valve replacement at the time of closure of the atrial septal defect and four patients had closure alone, one of whom required mitral valve replacement after five years. Three patients did not undergo closure of the atrial septal defect or mitral valve replacement because of severe coexisting medical problems. In six patients, the mitral valves were studied pathologically and all had thick, fibrotic leaflets and short, thick, fibrotic chordae tendineae. Three of these valves also had scattered areas of patchy myxomatous degeneration and three had areas of vascular ingrowth suggestive of rheumatic disease. Although both invasive and noninvasive studies have high-lighted the coincidence between atrial septal defect and mitral regurgitation, particularly the frequent association of mitral valve prolapse, our data indicate that this association rarely has clinical significance. Furthermore, the morphologic basis for mitral regurgitation in patients with atrial septal defect consists of leaflet and chordal thickening fibrosis and deformity rather than attenuation and ballooning as would be expected in mitral valve prolapse.     

Influence of gender on the etiology of mitral regurgitation

Our aim was to investigate the prevalence of the different causes of severe mitral regurgitation and the influence of gender on that prevalence. We performed a prospective study of 272 consecutive patients with severe mitral regurgitation that had been detected echocardiographically. Their mean age was 70.2 (13.8) years, and 143 were women (52.6%). Atrial fibrillation was present in 52.9%, 72.7% presented with heart failure, and 21.0% with previous myocardial infarction. The most common etiological factor was rheumatic disease (in 26.5%), with the etiology being unclear in 32 patients (11.8%). Rheumatic disease was more frequent in women, at 35.7%, than in men, at 16.3%, whereas other etiologies were less frequent in women (P< .001). In all age groups, a rheumatic etiology was more frequent in women. Rheumatic heart disease remains the main cause of severe mitral regurgitation observed in women referred to hospitals similar to ours.     

Prevalence of chronic rheumatic heart disease in Chinese adults

OBJECTIVES: Rheumatic heart disease continues to be a common health problem in the developing countries. Though there is evidence indicating that rheumatic heart disease prevalence in China has decreased since the 1950s, no objective assessment of its present prevalence has been published. The study was designed to investigate the prevalence of chronic rheumatic heart disease in China. METHODS: We performed a community population based investigation from October 2001 to February 2002 in nine communities of nine provinces in China by using a multistage, random sample design. Rheumatic heart disease was diagnosed by echocardiographic imaging. Long-axis views of the mitral valve, color flow recordings were used to search for mitral and aortic regurgitations. M-mode and two-dimensional short- and long-axis views of the aortic root and left atrium were recorded for supporting the diagnosis. RESULTS: Of 9124 participants, 8652 completed the questionnaires and 8080 had comprehensive echocardiographic examinations. We found that 15 subjects had definite echocardiographic evidence of rheumatic heart disease. The rough prevalence of rheumatic heart disease was 186/100,000 adults (2 in 1000 adults). CONCLUSIONS: Rheumatic heart disease affected approximately 2 million middle-aged to elderly Chinese, thus constituting a significant health burden. We investigated only urban and suburban communities, the result may underestimate the real prevalence of the disease in China.     

Real-time three-dimensional transthoracic echocardiography

BACKGROUND: Complex anatomy of intra-cardiac structures requires spatial orientation of image in three dimensions for better understanding and enhanced image interpretation. We evaluated the feasibility and efficacy of the emerging 'real-time three-dimensional transthoracic echocardiography' technique for comprehensive assessment of cardiac anatomy, physiology, pathomorphology and pathophysiology in patients with structural heart disease. METHODS AND RESULTS: Patients with structural heart disease (n=152) were evaluated by conventional two-dimensional transthoracic echocardiography and real-time three-dimensional transthoracic echocardiography using standard protocol. Fifty-six cases were of rheumatic etiology with multi-valvular involvement (mitral stenosis: 32; mitral regurgitation: 29; tricuspid regurgitation: 8; aortic valve disease: 11) and 21 cases of non-rheumatic valvular heart disease. A total of 38 congenital heart disease patients were examined including 23 patients with atrial septal defect. Left ventricular function (n=20) and right ventricular function (n=10) were also assessed using dedicated software. CONCLUSIONS: Results of real-time three-dimensional transthoracic echocardiography mitral valve area assessment by planimetery are comparable to two-dimensional transthoracic echocardiography with additional information about surface anatomy of leaflets and the subvalvular apparatus in real time with clear demarcation of commissural fusion and scallops of leaflets. Enface view of atrial septal defect with direct visualization of shape, size and number of defects, tricuspid valve area by planimetery, right ventricular shape, objective assessment of ventricular volumes and regurgitation vena contracta area are the fields where three-dimensional transthoracic echocardiography was of additive value to conventional two-dimensional transthoracic echcardiography. This study proves clinical feasibility of real-time three-dimensional transthoracic echocardiography but requires further validation of quantitative observations.     

Rheumatic fever and rheumatic heart disease in Japan

Rheumatic fever (RF) is the common cause of rheumatic heart disease (RHD) in the 5-30 age group and is usually preceded by group A streptococcal pharyngitis. The annual mortality caused by RF and RHD has changed remarkably in Japan between 1960 and 1981 according to the report of the Ministry of Health and Welfare. The annual incidence of RF among schoolchildren as surveyed from pediatric clinics of 20 major hospitals between 1952 and 1980 varied year by year, and is now steadily declining. Follow-up studies of 287 patients with carditis over ten years showed that cardiac murmur disappeared in 44.9% of total patients within 4 years through use of antibiotic prophylaxis. Other patients continued to have cardiac murmur 10 years after the first attack of RF. Valvular involvement due to RF was mostly confined to mitral valve and the appearance of mitral regurgitation was particularly common in schoolchildren. Survey of RHD in schoolchildren showed that mitral regurgitation was the most common anomaly, but the prevalence rate of RHD decreased from 4.6/1000 population in 1958 to 0.14/1000 population in 1981. By contrast, mitral stenosis was predominant in adults, according to the records of patients admitted in three university hospitals. Since socio-economic conditions have improved, and penicillin has been introduced to control group A streptococcus, mortality and morbidity from RF and RHD have remarkably decreased in well developed countries. These factors may have contributed predominantly to the sharp decrease in first and recurrent attack of RF in Japan.     

Occurrence and pattern of congenital heart diseases in a rural area of sub-Saharan Africa

Summary

The extent of congenital heart disease in Cameroon remains largely unknown. The aim of this study was to determine the occurrence and pattern of congenital heart diseases in the Cardiac Centre of St Elizabeth Catholic General Hospital, situated in a rural area of Cameroon.

Methods

Between November 2002 and November 2008, a population of 2 123 patients with suspected cardiac pathologies were consulted at St Elizabeth Catholic General Hospital referral cardiac centre. Of these patients, 292 subjects were recruited for the study, based on detection of (1) precordial murmurs and/or cardiomegaly on chest X-ray examination, or (2) congenital heart diseases on transthoracic Doppler echocardiography examination.

Results

Congenital heart diseases and inorganic murmurs were found in 95.5 and 4.5% of the patients, respectively. Congenital heart diseases included tetralogy of Fallot (26.1%), isolated ventricular septal defect (38.8%), atrioventricular cushion defect (7.3%), isolated atrial septal defect (2.8%), arterial duct cases (12.4%), common arterial trunk (1.3%), isolated stenosis of the pulmonary artery (2.6%), coarctation of the aorta (1.1%), congenital mitral valve regurgitation (1.2%), atresia of the triscupid valve (1.6%), double-outlet right ventricle (2.1%), anomalous pulmonary venous return (1.5%) and left isomerism (1.2%).

Conclusion

Our data show that there is a high occurrence of congenital heart disease in this hospital in a rural zone of sub-Saharan Africa and that isolated ventricular septal defect is the predominant pathology. Post-surgical follow up remains very challenging as many parents cannot afford their children’s medical treatment or are generally not well educated.     

Anesthesia for pregnant women with Mitral stenosis

Background Rheumatic heart disease is still a major heart problem associated with pregnancy in south China, despite its declining trend. The mortality and morbidity are considerably reduced1 by better perinatal care, where the anesthesiologist plays a major role in the multidisciplinary approach. Nevertheless, in wealthy nation, rheumatic disease has become uncommon and complex congenital heart disease is increasing in the recent decades. With the advent of intensive obstetric and anesthetic care, the death rate of pregnant women with heart disease is lower in mitral stenosis compared with other congenital heart diseases like Eisenmenger＇s syndrome, pulmonary vascular obstructive disease and Marfan＇s syndrome with aortopathy2. Although mitral stenosis is often associated with mitral regurgitation, morbidity is usually related to mitral stenosis3.     

Syndromes and malformations associated with congenital heart disease in a population-based study

Congenital cardiac malformations are frequently associated with non-cardiac malformations and chromosomal anomalies. Management is therefore influenced by interventional needs for all of the various anomalies. We have studied the association of congenital heart disease with extracardiac anomalies in the relatively closed population of Malta, where echocardiographic screening of all syndromic/multiply malformed infants is routinely carried out. Malformations were classified by using the EUROCAT method, for the first time. During 1990-1994, the birth prevalence of congenital heart disease was 8.8/1000 live births (n = 231). Of these, 21 (9%) had recognised chromosomal anomalies (0.80/1000 live births; 95% CI: 0.51-1.25), four (2%) had recognised non-chromosomal syndromes and 14 (6%) had other, major, non-cardiac malformations (0.69/1000 live births; 95% CI: 0.42-1.11). The commonest non-cardiac anomalies were musculoskeletal anomalies. Down syndrome accounted for 95% of all syndromic congenital heart disease, with a birth prevalence of 0.73/1000 live births (95% CI: 0.45-1.16). Comparison of these results with earlier studies showed wide disparities between studies, and this was attributed to differences in methods. such as differing inclusion criteria for both congenital heart disease and syndromes and malformations. The commonest lesion found in association with Down syndrome was isolated ventricular septal defect, not atrioventricular septal defect, and this was attributed to our screening process which identifies small lesions which would otherwise have been clinically missed and/or closed spontaneously.     

Valve repair in rheumatic heart disease in pediatric age group

Valve repair in children is technically demanding but more desirable than valve replacement. From April 2004 to September 2005, 1 boy and 8 girls with rheumatic heart disease, aged 2-13 years (median, 9 years), underwent valve repair for isolated mitral regurgitation in 5, combined mitral and aortic regurgitation in 2, mitral stenosis in 1, and mitral regurgitation associated with atrial septal defect in 1. Chordal shortening in 7, annular plication in 6, commissurotomy in 1, reconstruction of commissural leaflets in 7 were performed for mitral valve disease. Plication and reattachment of the aortic cusps was carried out in 2 patients. Annuloplasty rings were not used. All patients survived the operation, 8 had trivial or mild residual mitral regurgitation, and 1 had trivial aortic regurgitation. Mean left atrial pressure decreased from 14 to 7 mm Hg postoperatively. During follow-up of 3-18 months, all children were asymptomatic and enjoyed normal activity. None required reoperation. In addition to chordal shortening and annular plication, reconstruction of the commissural leaflets is considered the most important aspect of valve repair. It can be achieved without annuloplasty rings, giving good early and midterm results.     

Rheumatic heart disease: a school survey in a rural area of Rajasthan.

Thirty four of 10168 school going children were found to have rheumatic heart disease (RHD) in a school survey conducted in the rural areas of Churu district in Rajasthan. The prevalence rate was 3.34/1000. A high prevalence of RHD was found in 11 to 15 year age group. Maximum prevalence of RHD was in low socioeconomic group. Out of these thirty four cases of RHD, isolated mitral stenosis was present in sixteen, isolated mitral regurgitation in four, combined mitral stenosis and mitral regurgitation in ten patients.     

Atrial septal defect in adult identical twins: A variation in theme

The diagnosis of atrial septal defect was established in monozygotic twin females at age 63. Each patient mimicked a different acquired heart disease. One twin had congestive heart failure and atrial fibrillation and was diagnosed as having rheumatic mitral insufficiency. The other twin had atypical chest pain and systemic hypertension and was thought to have arteriosclerotic heart disease. In each case the correct diagnosis was made at cardiac catheterization. Although the same basic congenital heart lesion was present in both patients, the symptoms and findings differed. Symptomatic improvement was achieved by different therapeutic modalities. One patient had open heart surgery, while the other twin improved with medical therapy.     

Etiology and pathogenesis of thromboembolism.

Thrombus formation in the left atrium and left ventricle is primarily due to stasis of blood which causes activation of the coagulation system. Migration of thrombotic material into the circulation depends on the dynamic forces of the circulation. Atrial fibrillation is the commonest underlying cardiac disorder predisposing to thromboembolism. Rheumatic mitral stenosis, left atrial enlargement, prior myocardial infarction, hypertension, and echocardiographic left ventricular hypertrophy are risk factors for thromboembolic stroke in elderly patients with chronic atrial fibrillation. Non-valvular atrial fibrillation accounts for 45% of cardiac sources of thromboembolic stroke and includes patients with ischemic heart disease, hypertension, thyrotoxic heart disease, hypertrophic cardiomyopathy, chronic sinoatrial disorder, and idiopathic atrial fibrillation. 15% of cardiac sources of thromboembolic stroke are associated with acute myocardial infarction, 10% with left ventricular aneurysm and mural thrombi remote from an acute myocardial infarction, 10% with rheumatic valvular heart disease, and 10% with prosthetic cardiac valves. Mitral valve prolapse, mitral annular calcium, nonischemic cardiomyopathies, infective endocarditis, nonbacterial thrombotic endocarditis, left atrial myxoma, paradoxical embolism associated with congenital heart disease, calcific aortic stenosis, and complex atherosclerotic plaque within the proximal aorta also contribute to thromboembolism.     

Two-dimensional Doppler color flow mapping in congenital heart disease

The development of a system that images intracardiac anatomy cross-sectionally while simultaneously displaying intracardiac blood flow in realtime has the potential of increasing diagnostic capabilities in the evaluation of congenital heart lesions. This would translate into the ability to visualize intracardiac lesions not identified by standard M-mode, two dimensional (2-D), pulsed, and continuous wave Doppler modalities. A review of 1000 consecutive studies in our laboratory was performed. Doppler color flow mapping was performed in each case after complete segmental analysis by 2-D and Doppler echo. Identification of intracardiac lesions or abnormalities of blood flow by Doppler color flow mapping, not appreciated on routine 2-D/Doppler studies included ventricular septal defect (VSD) (n = 24), atrial septal defect (n = 4), mitral regurgitation (n = 13), tricuspid regurgitation after VSD closure (n = 5), tricuspid regurgitation in association with atrial septal defect (ASD) (n = 7), residual ventricular septal defect after surgery (n = 10), pulmonary insufficiency (n = 4), aortic insufficiency (n = 4), and patent Blalock-Taussig shunt (n = 2). Identification of multiple ventricular septal defects in two patients and the presence of a large left-to-right shunt across an atrial septal defect in the presence of septal aneurysm formation was also aided by Doppler color flow. Use of color flow to identify areas of maximal velocity and turbulence greatly enhanced continuous wave Doppler measurements by directing placement of the Doppler beam within the flow areas.     

Spread of heart diseases seen in an open-access paediatric echocardiography clinic

All patients <13 years of age referred to the open-access paediatric echocardiography clinic at the Sultan Qaboos University Hospital, Muscat, Oman, during the five years from 1994 to 1998 were analysed. Among the 2633 patients studied, 1543 (58.6%) were normal, 845 (32.1%) had congenital heart disease, and 245 (9.3%) had acquired heart disease. The major congenital heart diseases identified were secundum atrial septal defect (22.5%), ventricular septal defect (22.5%), patent ductus arteriosus (15.7%), mitral valve prolapse (10.7%), pulmonary stenosis (9.7%) and atrioventricular septal defect (4.5%). Fifty-eight percent of the congenital heart diseases were identified in the first year of life. Among the acquired heart diseases, rheumatic heart disease (30.2%) and cardiac involvement secondary to haemoglobinopathies (16.7%), dilated cardiomyopathy (16.3%) and hypertrophic cardiomyopathy (12.7%) were significant. Although the presence of specific cardiac symptoms was associated with a high yield of abnormalities, such disorders were also discovered in a significant number of children with isolated cardiac murmur. The referral source did not influence significantly the frequency of heart diseases diagnosed in this study. Open-access echocardiography is important in early detection of heart disease in the paediatric population.     

An autopsy case of endocardial cushion defect (ECD) in an 82-year-old female]

In an 82-year-old female case of endocardial cushion defect (ECD), a systolic regurgitant murmur was heard at the apex, and her ECG showed atrial fibrillation without right bundle branch block or left axis deviation. An echocardiogram demonstrated atrial septal defect (ASD) and a cleft of the anterior mitral leaflet with calcification. She died of refractory congestive heart failure. Autopsy revealed ECD (intermediate type) with mitral and tricuspid cleft, and ASD (ostium primum type, 2.0 x 1.0 cm in diameter). In addition, mitral ring calcification and calcification of the cleft mitral valve was disclosed, causing mitral stenosis in addition to mitral regurgitation due to the cleft mitral valve. This was the second oldest Japanese autopsy case of ECD. We concluded that echocardiographic examinations, including color flow imaging, in aged patients with heart murmur are necessary to confirm the diagnosis of congenital heart disease in the aged.